Approved Treatments for Alport Syndrome

Alport syndrome is an inherited disorder that is characterized by progressive kidney damage, hearing problems, and eye abnormalities. There is currently no cure for Alport syndrome and treatments are limited, but research is ongoing and various therapies are used to help patients manage its symptoms.

Most of the medications used in Alport syndrome aim to slow the progression of kidney damage. The most commonly prescribed medicines are angiotensin-converting enzyme (ACE) inhibitors. These reduce the levels of the enzyme angiotensin II that acts to narrow blood vessels and increase blood pressure.

ACE Inhibitors

Angiotensin-converting-enzyme (ACE) inhibitors, which help to relax blood vessels, are medications to treat high blood pressure. They are used to slow the damage that can lead to kidney failure and improve life expectancy in Alport syndrome patients. They also can reduce proteinuria, the presence of proteins in the urine, in these patients. Proteinuria is associated with kidney damage.

ACE inhibitors may be used in combination with angiotensin receptor blockers (ARBs).

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Accupril is used to treat high blood pressure and heart failure. This medication inhibits the elevation of blood pressure caused by angiotensin I. Accupril promotes the retention of potassium. Potassium-rich foods, such as dairy products, salt substitutes, nuts, and certain fruits and vegetables should be avoided while taking this medication.

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Aceon is used to treat high blood pressure and coronary artery disease and is among the ACE inhibitors recommended as first-line treatments. Perindopril, the active ingredient of Aceon, is a pro-drug; once ingested, it is metabolized by the liver to its active form, which is called perindoprilat. Perindoprilat competes for the active site of ACE. ACE enzymes that are bound to perindoprilat are not available to bind to angiotensin 1 and convert it to angiotensin 2.

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Altace is an approved treatment for high blood pressure (hypertension), and to prevent related heart and kidney damage. It is thought to protect a patient’s kidneys mainly by lowering the glomerular filtration pressure and blocking the local angiotensin-system at podocytes, a group of highly specialized cells in the kidneys. This results in a reduction in proteinuria and excessive kidney fibrosis (the formation of hardened and scarred tissue).

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Cilazapril is used to treat high blood pressure and can be used to delay the onset of kidney failure. It is not available in the U.S., but it is used in Canada and parts of Europe. As a pro-drug, once ingested it is metabolized by the liver to produce another drug, which competes for the active site of ACE. ACE that becomes bound to cilazaprilat is not available to bind angiotensin 1 and convert it to angiotensin 2.

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Enalapril treats high blood pressure in adults and children one month or older. Enalapril is also used to treat congestive heart failure in adults and to delay the onset of kidney failure in people with Alport syndrome. The metabolized form of the treatment competes for the active site of the ACE enzyme and therefore stops it from binding to angiotensin 1 and converting it to angiotensin 2.

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Lotensin is approved to treat high blood pressure and also may be used to reduce a strain on the kidneys in patients with Alport syndrome. It can be used in combination with other medications to halt the disease progression in patients who are not in kidney failure before the start of treatment. A generic form of the treatment also is available from several companies.

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Mavik is approved to treat high blood pressure and congestive heart failure. It is also used to treat some patients after a heart attack. This medication may also be prescribed off-label to people with Alport syndrome as a first-line therapy to treat proteinuria and kidney disease.

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Monopril is the sodium salt of fosinopril, used to treat high blood pressure and heart failure. The active form, fosinoprilat, is an inhibitor of ACE. Patients given Monopril also have a lower incidence of cardiovascular mortality and hospitalization for cardiovascular disease.

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Other Inhibitors

Aldosterone inhibitors are a class of medications that affect the secretion of fluid and the retention of salt in the kidneys. By blocking the binding of aldosterone to its receptor, the amount of water and sodium excreted by the kidneys is increased, and potassium is retained. This class of medication often is prescribed to control blood pressure.

Angiotensin receptor blockers (ARBs) are medications used to lower blood pressure.

Angiotensin Receptor Blockers

ARBs work by blocking the receptors to which angiotensin normally binds. As a result, blood pressure decreases. They can be taken in combination with ACE inhibitors or used as an alternative in patients who cannot tolerate these medications.

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Aldactone is an aldosterone inhibitor available by prescription from many pharmaceutical companies. It is approved to treat conditions that cause fluid retention, or edema, such as congestive heart failure, hypertension, and kidney disease. It can also be used as a kidney-protective therapy in Alport syndrome.

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Inspra is an aldosterone inhibitor that is used to treat high blood pressure and congestive heart failure after a heart attack. In Alport syndrome patients, it is used to lower blood pressure and to treat proteinuria. Inspra is recommended as a second-line treatment for Alport in combination with ACE inhibitors.

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